Investig Magn Reson Imaging.  2021 Dec;25(4):332-337. 10.13104/imri.2021.25.4.332.

Cardiac Behçet's Disease Presenting with Right Ventricular Endomyocardial Fibrosis and Intracardiac Thrombosis: a Case Report

Affiliations
  • 1Department of Radiology and Research Institute of Radiology, Asan Medical Center, Seoul, Korea
  • 2Division of Cardiology, Cardiothoracic Imaging Center, Asan Medical Center, Seoul, Korea
  • 3Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea

Abstract

Behçet’s disease is a chronic inflammatory disorder involving vessels of various sizes and organs, including the skin, joints, gastrointestinal tract, lungs, and cardiovascular system. The etiology of Behçet’s disease is unclear, and clinical diagnosis is important in the absence of definitive laboratory or pathological findings diagnostic of Behçet’s disease. Cardiac involvement is rare but might present as endocarditis, myocarditis, pericarditis, or intracardiac thrombosis. This report presents a case of Behçet’s disease involving the heart in a 22-year-old man with unusual manifestations of right ventricular fibrosis and intracardiac thrombosis. Cardiac magnetic resonance imaging revealed multiple intracardiac thrombi and delayed diffuse subendocardial enhancement involving the right ventricle. No peripheral eosinophilia was detected. Endomyocardial biopsy showed mixed inflammatory cell infiltrates. Based on the patient’s clinical history of oral ulcer and arthritis, a diagnosis of Behçet’s disease was made considering the clinical, radiological, and histological findings. Intracardiac thrombi and endomyocardial fibrosis are rare manifestations of Behçet’s disease, and the diagnosis is often a clinical challenge. Early diagnosis is important for appropriate management. Behçet’s disease should be considered in the differential diagnosis of patients with intracardiac thrombosis and endomyocardial fibrosis of the right chamber.

Keyword

Behçet’s disease; Intracardiac thrombosis; Endomyocardial fibrosis; Magnetic resonance imaging
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